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COL4A3 recombinant protein :: Collagen Type IV Alpha 3 Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS142942 COL4A3 recombinant protein
Unit / Price
0.002 mg  /  $140 +1 FREE 8GB USB
0.01 mg  /  $205 +1 FREE 8GB USB
1 mg  /  $5,015 +6 FREE 8GB USB
 
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 Product Name   

Collagen Type IV Alpha 3 (COL4A3), Recombinant Protein

★Popular Item★
 Also Known As   

Recombinant Human Collagen Type IV Alpha 3; COL4A3 Human

 Product Synonym Names    Collagen alpha-3(IV) chain; Goodpasture antigen; COL4A3; Glomerular Basal Membrane; GBM
 Product Gene Name   

COL4A3 recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    104200
 3D Structure    ModBase 3D Structure for Q01955
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 Form/Format    The protein solution (0.6 mg/ml) contains 20mM Hepes, pH 8.0, and 4M Urea
 Concentration    0.6 mg/ml (lot specific)
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 Coating Concentration    0.12-0.5 ug/ml (depending on the type of ELISA plate and coating buffer). Suitable for biotinylation and iodination.
 Source    Sf9 Insect Cells
 Physical Appearance    Sterile Filtered clear solution.
 Caution    It has been reported that the immunodominant epitope of COL4A3 is a cryptic epitope that is not easily accessible to the corresponding autoantibodies. It is necessary to treat the protein under nonreducing conditions with a denaturant such as urea to unmask the epitopes (see Hellmark et al. in Autoantibodies, Peter, J.B. and Shoenfeld, Y., eds., Elsevier B.V., 1996, pp 291-298).
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 Preparation and Storage    Recommendations for storage buffer: Ionic strength between 50 and 100 mM, neutral to slightly alkaline pH and 4 M urea as dissociating agent.
Storage temperature is -70 degree to -80 degree C.
Please prevent freeze-thaw cycles.
 Other Notes    Small volumes of COL4A3 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for COL4A3 recombinant protein

   Description: Human a3 chain of collagen IV; identical with the antigen called "glomerular basal membrane antigen" (GBM). Recombinant antigen for solid (ELISA) and fluid phase diagnostic assays. Binds IgG-type human auto-antibodies. Calculated Molecular weight: 43,591 Dalton. Calculated isoelectric point: pH 8.9. cDNA coding for a minicollagen version of the human collagen IV a3 chain fused to a hexa-histidine purification tag. The term minicollagen designates the removal of most of the epitope-less triplehelical collagenous region (situated between the N-terminal 7S domain and the C-terminal noncollagenous NC1 domain), which is a requirement for recombinant production of this antigen.

Introduction: Type IV collagen is a major structural component of basement membranes. It is a multimeric protein composed of 3 alpha subunits, which are encoded by 6 different genes, alpha 1 through alpha 6. Each of these alpha subunits can form a triple helix structure with 2 other subunits to form type IV collagen. The Goodpasture syndrome is a condition in which autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are restricted basically to the non-collagenous C-terminal domain of the protein. There are numerous alternative transcripts that appear to be unique to the human COL4A3 and alternative splicing is limited to the six exons that encode this C-terminal domain. COL4A3 is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to the Alport syndrome are also situated within the exons that encode this C-terminal region. COL4A3 is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter. Several exons of COL4A3 are interspersed with exons of an uncharacterized gene which is on the opposite strand.
 Product Categories/Family for COL4A3 recombinant protein    RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins; Collagen
NCBI/Uniprot data below describe general gene information for COL4A3. It may not necessarily be applicable to this product.
 NCBI GI #    89142730
 NCBI GeneID    1285
 NCBI Accession #    NP_000082.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_000091.4 [Other Products]
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 UniProt Primary Accession #    Q01955 [Other Products]
 UniProt Secondary Accession #    Q53QQ1; Q53R14; Q53RW8; Q9BQT2; Q9NYC4; Q9UDJ9; Q9UDK9; Q9UDL0; Q9UDL1 [Other Products]
 UniProt Related Accession #    Q01955 [Other Products]
 Molecular Weight    135,079 Da
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 NCBI Official Full Name    collagen alpha-3(IV) chain
 NCBI Official Synonym Full Names    collagen, type IV, alpha 3 (Goodpasture antigen)
 NCBI Official Symbol    COL4A3 [Similar Products]
 NCBI Protein Information    collagen alpha-3(IV) chain; collagen alpha-3(IV) chain; collagen IV, alpha-3 polypeptide; tumstatin
 UniProt Protein Name    Collagen alpha-3(IV) chain
 UniProt Synonym Protein Names   
Goodpasture antigenTumstatin
 Protein Family    Collagen
 UniProt Gene Name    COL4A3 [Similar Products]
 UniProt Entry Name    CO4A3_HUMAN
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 NCBI Summary for COL4A3    Type IV collagen, the major structural component of basement membranes, is a multimeric protein composed of 3 alpha subunits. These subunits are encoded by 6 different genes, alpha 1 through alpha 6, each of which can form a triple helix structure with 2 other subunits to form type IV collagen. This gene encodes alpha 3. In the Goodpasture syndrome, autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are localized largely to the non-collagenous C-terminal domain of the protein. A specific kinase phosphorylates amino acids in this same C-terminal region and the expression of this kinase is upregulated during pathogenesis. This gene is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to this syndrome are also located within the exons that encode this C-terminal region. Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter. [provided by RefSeq, Jun 2010]
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 UniProt Comments for COL4A3    COL4A3: Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. Autoantibodies against the NC1 domain of alpha 3(IV) are found in Goodpasture syndrome, an autoimmune disease of lung and kidney. Defects in COL4A3 are a cause of Alport syndrome autosomal recessive (APSAR). APSAR is characterized by progressive glomerulonephritis, glomerular basement membrane defects, renal failure, sensorineural deafness and specific eye abnormalities (lenticonous and macular flecks). The disorder shows considerable heterogeneity in that families differ in the age of end-stage renal disease and the occurrence of deafness. Defects in COL4A3 are a cause of benign familial hematuria (BFH); also known as thin basement membrane nephropathy. BFH is characterized by persistent hematuria, an electron microscopically detectable thin glomerular basement membrane (GBM) and an autosomal dominant mode of inheritance. Renal function remains normal. In children, differentiation between BFH and AS can be difficult, because both disorders are manifested by persistent hematuria and thin GBM at that age. Defects in COL4A3 are a cause of Alport syndrome autosomal dominant (APSAD). Alport syndrome is characterized by progressive glomerulonephritis, glomerular basement membrane defects, renal failure, sensorineural deafness and specific eye abnormalities (lenticonous and macular flecks). The disorder shows considerable heterogeneity in that families differ in the age of end-stage renal disease and the occurrence of deafness. Belongs to the type IV collagen family. 5 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 2q36-q37

Cellular Component: endoplasmic reticulum lumen; collagen type IV; extracellular region; basement membrane

Molecular Function: metalloendopeptidase inhibitor activity; integrin binding; protein binding; extracellular matrix structural constituent; structural molecule activity

Biological Process: caspase activation; axon guidance; extracellular matrix organization and biogenesis; blood circulation; glomerular basement membrane development; cell proliferation; extracellular matrix disassembly; collagen catabolic process; negative regulation of cell proliferation; negative regulation of angiogenesis; sensory perception of sound; cell surface receptor linked signal transduction; cell adhesion

Disease: Hematuria, Benign Familial; Alport Syndrome, Autosomal Dominant; Alport Syndrome, Autosomal Recessive
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 Research Articles on COL4A3    1. Release of recombinant tumstatin in transgenic platelet granules led to antiangiogenic effect of platelets.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with COL4A3 recombinant proteinPathways associated with COL4A3 recombinant protein
 Reference Product  PubMed Publications
 COL4A4 recombinant protein  >146 publications with COL4A3 and COL4A4
 COL4A5 recombinant protein  >96 publications with COL4A3 and COL4A5
 COL4A6 recombinant protein  >12 publications with COL4A3 and COL4A6
 COL6A3 recombinant protein  >1 publications with COL4A3 and COL6A3
 COL6A1 recombinant protein  >1 publications with COL4A3 and COL6A1
 Products by Pathway  Pathway Diagram
 Amoebiasis Pathway antibodies  Amoebiasis Pathway Diagram
 Amoebiasis Pathway antibodies  Amoebiasis Pathway Diagram
 Assembly Of Collagen Fibrils And Other Multimeric Structures Pathway antibodies  Assembly Of Collagen Fibrils And Other Multimeric Structures Pathway Diagram
 Axon Guidance Pathway antibodies  Axon Guidance Pathway Diagram
 Collagen Biosynthesis And Modifying Enzymes Pathway antibodies  Collagen Biosynthesis And Modifying Enzymes Pathway Diagram
 Collagen Formation Pathway antibodies  Collagen Formation Pathway Diagram
 Developmental Biology Pathway antibodies  Developmental Biology Pathway Diagram
 ECM-receptor Interaction Pathway antibodies  ECM-receptor Interaction Pathway Diagram
 ECM-receptor Interaction Pathway antibodies  ECM-receptor Interaction Pathway Diagram
 Extracellular Matrix Organization Pathway antibodies  Extracellular Matrix Organization Pathway Diagram
Diseases associated with COL4A3 recombinant proteinOrgans/Tissues associated with COL4A3 recombinant protein
 Disease Name  Pubmed Publications
 Kidney Diseases Antibodies  >163 publications with COL4A3 and Kidney Diseases
 Nephritis, Hereditary Antibodies  >119 publications with COL4A3 and Nephritis, Hereditary
 Urogenital Abnormalities Antibodies  >114 publications with COL4A3 and Urogenital Abnormalities
 Proteinuria Antibodies  >46 publications with COL4A3 and Proteinuria
 Hemorrhage Antibodies  >36 publications with COL4A3 and Hemorrhage
 Fibrosis Antibodies  >22 publications with COL4A3 and Fibrosis
 Immune System Diseases Antibodies  >19 publications with COL4A3 and Immune System Diseases
 Nervous System Diseases Antibodies  >14 publications with COL4A3 and Nervous System Diseases
 Respiratory Tract Diseases Antibodies  >13 publications with COL4A3 and Respiratory Tract Diseases
 Inflammation Antibodies  >11 publications with COL4A3 and Inflammation
 Organ/Tissue Name  Pubmed Publications
 Kidney Antibodies  >142 publications with COL4A3 and Kidney
 Eye Antibodies  >18 publications with COL4A3 and Eye
 Muscle Antibodies  >11 publications with COL4A3 and Muscle
 Vascular Antibodies  >11 publications with COL4A3 and Vascular
 Connective Tissue Antibodies  >10 publications with COL4A3 and Connective Tissue
 Lung Antibodies  >4 publications with COL4A3 and Lung
 Liver Antibodies  >3 publications with COL4A3 and Liver
 Testis Antibodies  >1 publications with COL4A3 and Testis
 Brain Antibodies  >1 publications with COL4A3 and Brain
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